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Memantine Protects From Exacerbation of Ischemic Stroke and Blood Brain Barrier Disruption in Mild But Not Severe Hyperhomocysteinemia.

Gu SX, Sonkar VK, Katare PB, Kumar R, Kruger WD, Arning E, Bottiglieri T, Lentz SR, Dayal S.

J Am Heart Assoc. 2020 Feb 18;9(4):e013368. doi: 10.1161/JAHA.119.013368. Epub 2020 Feb 13.


Analysis of the Qatari R336C cystathionine β-synthase protein in mice.

Gupta S, Gallego-Villar L, Wang L, Lee HO, Nasrallah G, Al-Dewik N, Häberle J, Thöny B, Blom HJ, Ben-Omran T, Kruger WD.

J Inherit Metab Dis. 2019 Sep;42(5):831-838. doi: 10.1002/jimd.12140. Epub 2019 Jul 10.


Treatment of Cystathionine β-Synthase Deficiency in Mice Using a Minicircle-Based Naked DNA Vector.

Lee HO, Gallego-Villar L, Grisch-Chan HM, Häberle J, Thöny B, Kruger WD.

Hum Gene Ther. 2019 Sep;30(9):1093-1100. doi: 10.1089/hum.2019.014. Epub 2019 Jun 13.


In silico and in vivo models for Qatari-specific classical homocystinuria as basis for development of novel therapies.

Ismail HM, Krishnamoorthy N, Al-Dewik N, Zayed H, Mohamed NA, Giacomo VD, Gupta S, Häberle J, Thöny B, Blom HJ, Kruger WD, Ben-Omran T, Nasrallah GK.

Hum Mutat. 2019 Feb;40(2):230-240. doi: 10.1002/humu.23682. Epub 2018 Nov 23.


Mouse modeling and structural analysis of the p.G307S mutation in human cystathionine β-synthase (CBS) reveal effects on CBS activity but not stability.

Gupta S, Kelow S, Wang L, Andrake MD, Dunbrack RL Jr, Kruger WD.

J Biol Chem. 2018 Sep 7;293(36):13921-13931. doi: 10.1074/jbc.RA118.002164. Epub 2018 Jul 20.


S-adenosylhomocysteine hydrolase over-expression does not alter S-adenosylmethionine or S-adenosylhomocysteine levels in CBS deficient mice.

Lee HO, Wang L, Kuo YM, Andrews AJ, Gupta S, Kruger WD.

Mol Genet Metab Rep. 2018 Jan 12;15:15-21. doi: 10.1016/j.ymgmr.2018.01.002. eCollection 2018 Jun.


Specific Targeting of MTAP-Deleted Tumors with a Combination of 2'-Fluoroadenine and 5'-Methylthioadenosine.

Tang B, Lee HO, An SS, Cai KQ, Kruger WD.

Cancer Res. 2018 Aug 1;78(15):4386-4395. doi: 10.1158/0008-5472.CAN-18-0814. Epub 2018 May 29.


Enzyme Replacement Therapy Ameliorates Multiple Symptoms of Murine Homocystinuria.

Majtan T, Jones W Jr, Krijt J, Park I, Kruger WD, Kožich V, Bassnett S, Bublil EM, Kraus JP.

Mol Ther. 2018 Mar 7;26(3):834-844. doi: 10.1016/j.ymthe.2017.12.014. Epub 2017 Dec 19.


Cysteamine revisited: repair of arginine to cysteine mutations.

Gallego-Villar L, Hannibal L, Häberle J, Thöny B, Ben-Omran T, Nasrallah GK, Dewik AN, Kruger WD, Blom HJ.

J Inherit Metab Dis. 2017 Jul;40(4):555-567. doi: 10.1007/s10545-017-0060-4. Epub 2017 Jun 22. Review.


Cystathionine β-synthase deficiency: Of mice and men.

Kruger WD.

Mol Genet Metab. 2017 Jul;121(3):199-205. doi: 10.1016/j.ymgme.2017.05.011. Epub 2017 May 19. Review.


The c.797 G>A (p.R266K) cystathionine β-synthase mutation causes homocystinuria by affecting protein stability.

Gupta S, Wang L, Kruger WD.

Hum Mutat. 2017 Jul;38(7):863-869. doi: 10.1002/humu.23240. Epub 2017 May 22.


Combination of serum histidine and plasma tryptophan as a potential biomarker to detect clear cell renal cell carcinoma.

Lee HO, Uzzo RG, Kister D, Kruger WD.

J Transl Med. 2017 Apr 6;15(1):72. doi: 10.1186/s12967-017-1178-8.


The amino acid metabolite homocysteine activates mTORC1 to inhibit autophagy and form abnormal proteins in human neurons and mice.

Khayati K, Antikainen H, Bonder EM, Weber GF, Kruger WD, Jakubowski H, Dobrowolski R.

FASEB J. 2017 Feb;31(2):598-609. doi: 10.1096/fj.201600915R. Epub 2016 Oct 25.


Homocysteine modulates 5-lipoxygenase expression level via DNA methylation.

Li JG, Barrero C, Gupta S, Kruger WD, Merali S, Praticò D.

Aging Cell. 2017 Apr;16(2):273-280. doi: 10.1111/acel.12550. Epub 2016 Nov 29.


Lack of global epigenetic methylation defects in CBS deficient mice.

Lee HO, Wang L, Kuo YM, Gupta S, Slifker MJ, Li YS, Andrews AJ, Kruger WD.

J Inherit Metab Dis. 2017 Jan;40(1):113-120. doi: 10.1007/s10545-016-9958-5. Epub 2016 Jul 21.


The effect of dietary modulation of sulfur amino acids on cystathionine β synthase-deficient mice.

Kruger WD, Gupta S.

Ann N Y Acad Sci. 2016 Jan;1363:80-90. doi: 10.1111/nyas.12967. Epub 2015 Nov 24. Review.


Betaine supplementation is less effective than methionine restriction in correcting phenotypes of CBS deficient mice.

Gupta S, Wang L, Kruger WD.

J Inherit Metab Dis. 2016 Jan;39(1):39-46. doi: 10.1007/s10545-015-9883-z. Epub 2015 Aug 1.


Hydroxychloroquine Destabilizes Phospho-S6 in Human Renal Carcinoma Cells.

Lee HO, Mustafa A, Hudes GR, Kruger WD.

PLoS One. 2015 Jul 2;10(7):e0131464. doi: 10.1371/journal.pone.0131464. eCollection 2015.


Expression of MTAP inhibits tumor-related phenotypes in HT1080 cells via a mechanism unrelated to its enzymatic function.

Tang B, Kadariya Y, Chen Y, Slifker M, Kruger WD.

G3 (Bethesda). 2014 Nov 11;5(1):35-44. doi: 10.1534/g3.114.014555.


Hyperhomocysteinemia potentiates hyperglycemia-induced inflammatory monocyte differentiation and atherosclerosis.

Fang P, Zhang D, Cheng Z, Yan C, Jiang X, Kruger WD, Meng S, Arning E, Bottiglieri T, Choi ET, Han Y, Yang XF, Wang H.

Diabetes. 2014 Dec;63(12):4275-90. doi: 10.2337/db14-0809. Epub 2014 Jul 9.


Protein arginine hypomethylation in a mouse model of cystathionine β-synthase deficiency.

Esse R, Imbard A, Florindo C, Gupta S, Quinlivan EP, Davids M, Teerlink T, Tavares de Almeida I, Kruger WD, Blom HJ, Castro R.

FASEB J. 2014 Jun;28(6):2686-95. doi: 10.1096/fj.13-246579. Epub 2014 Feb 14. Erratum in: FASEB J. 2015 Mar;29(3):1125.


Quantitation of cellular metabolic fluxes of methionine.

Shlomi T, Fan J, Tang B, Kruger WD, Rabinowitz JD.

Anal Chem. 2014 Feb 4;86(3):1583-91. doi: 10.1021/ac4032093. Epub 2014 Jan 16.


Cystathionine β-synthase-deficient mice thrive on a low-methionine diet.

Gupta S, Melnyk SB, Kruger WD.

FASEB J. 2014 Feb;28(2):781-90. doi: 10.1096/fj.13-240770. Epub 2013 Nov 4.


Germline Mutations in Mtap Cooperate with Myc to Accelerate Tumorigenesis in Mice.

Kadariya Y, Tang B, Wang L, Al-Saleem T, Hayakawa K, Slifker MJ, Kruger WD.

PLoS One. 2013 Jun 26;8(6):e67635. doi: 10.1371/journal.pone.0067635. Print 2013.


Correction of cystathionine β-synthase deficiency in mice by treatment with proteasome inhibitors.

Gupta S, Wang L, Anderl J, Slifker MJ, Kirk C, Kruger WD.

Hum Mutat. 2013 Aug;34(8):1085-93. doi: 10.1002/humu.22335. Epub 2013 May 13.


The TSC1/2 complex controls Drosophila pigmentation through TORC1-dependent regulation of catecholamine biosynthesis.

Zitserman D, Gupta S, Kruger WD, Karbowniczek M, Roegiers F.

PLoS One. 2012;7(11):e48720. doi: 10.1371/journal.pone.0048720. Epub 2012 Nov 7.


Increasing the therapeutic index of 5-fluorouracil and 6-thioguanine by targeting loss of MTAP in tumor cells.

Tang B, Testa JR, Kruger WD.

Cancer Biol Ther. 2012 Sep;13(11):1082-90. doi: 10.4161/cbt.21115. Epub 2012 Jul 24.


Severe hyperhomocysteinemia promotes bone marrow-derived and resident inflammatory monocyte differentiation and atherosclerosis in LDLr/CBS-deficient mice.

Zhang D, Fang P, Jiang X, Nelson J, Moore JK, Kruger WD, Berretta RM, Houser SR, Yang X, Wang H.

Circ Res. 2012 Jun 22;111(1):37-49. doi: 10.1161/CIRCRESAHA.112.269472. Epub 2012 May 24.


Brain phenotype of transgenic mice overexpressing cystathionine β-synthase.

Régnier V, Billard JM, Gupta S, Potier B, Woerner S, Paly E, Ledru A, David S, Luilier S, Bizot JC, Vacano G, Kraus JP, Patterson D, Kruger WD, Delabar JM, London J.

PLoS One. 2012;7(1):e29056. doi: 10.1371/journal.pone.0029056. Epub 2012 Jan 12.


Paradoxical absence of a prothrombotic phenotype in a mouse model of severe hyperhomocysteinemia.

Dayal S, Chauhan AK, Jensen M, Leo L, Lynch CM, Faraci FM, Kruger WD, Lentz SR.

Blood. 2012 Mar 29;119(13):3176-83. doi: 10.1182/blood-2011-09-380568. Epub 2011 Dec 20.


Cystathionine beta-synthase deficiency causes fat loss in mice.

Gupta S, Kruger WD.

PLoS One. 2011;6(11):e27598. doi: 10.1371/journal.pone.0027598. Epub 2011 Nov 11.


Serum amino acid levels as a biomarker for renal cell carcinoma.

Mustafa A, Gupta S, Hudes GR, Egleston BL, Uzzo RG, Kruger WD.

J Urol. 2011 Oct;186(4):1206-12. doi: 10.1016/j.juro.2011.05.085. Epub 2011 Aug 17.


Inhibition of betaine-homocysteine S-methyltransferase in rats causes hyperhomocysteinemia and reduces liver cystathionine β-synthase activity and methylation capacity.

Strakova J, Gupta S, Kruger WD, Dilger RN, Tryon K, Li L, Garrow TA.

Nutr Res. 2011 Jul;31(7):563-71. doi: 10.1016/j.nutres.2011.06.004.


Hyperhomocysteinemia impairs endothelium-derived hyperpolarizing factor-mediated vasorelaxation in transgenic cystathionine beta synthase-deficient mice.

Cheng Z, Jiang X, Kruger WD, Praticò D, Gupta S, Mallilankaraman K, Madesh M, Schafer AI, Durante W, Yang X, Wang H.

Blood. 2011 Aug 18;118(7):1998-2006. doi: 10.1182/blood-2011-01-333310. Epub 2011 Jun 8.


Chemical genetic screening for compounds that preferentially inhibit growth of methylthioadenosine phosphorylase (MTAP)-deficient Saccharomyces cerevisiae.

Kadariya Y, Tang B, Myers CB, Fukui J, Peterson JR, Kruger WD.

J Biomol Screen. 2011 Jan;16(1):44-52. doi: 10.1177/1087057110386371. Epub 2010 Dec 3.


Dietary intake of S-(alpha-carboxybutyl)-DL-homocysteine induces hyperhomocysteinemia in rats.

Strakova J, Williams KT, Gupta S, Schalinske KL, Kruger WD, Rozen R, Jiracek J, Li L, Garrow TA.

Nutr Res. 2010 Jul;30(7):492-500. doi: 10.1016/j.nutres.2010.06.017.


Testing computational prediction of missense mutation phenotypes: functional characterization of 204 mutations of human cystathionine beta synthase.

Wei Q, Wang L, Wang Q, Kruger WD, Dunbrack RL Jr.

Proteins. 2010 Jul;78(9):2058-74. doi: 10.1002/prot.22722.


The Herp protein pathway is not involved in the pro-amyloidogenic effect of hyperhomocysteinemia.

Zhuo JM, Kruger WD, Praticò D.

J Alzheimers Dis. 2010;20(2):569-76. doi: 10.3233/JAD-2010-1394.


Activation of mutant enzyme function in vivo by proteasome inhibitors and treatments that induce Hsp70.

Singh LR, Gupta S, Honig NH, Kraus JP, Kruger WD.

PLoS Genet. 2010 Jan;6(1):e1000807. doi: 10.1371/journal.pgen.1000807. Epub 2010 Jan 8.


Methionine-deficient diet induces post-transcriptional downregulation of cystathionine β-synthase.

Tang B, Mustafa A, Gupta S, Melnyk S, James SJ, Kruger WD.

Nutrition. 2010 Nov-Dec;26(11-12):1170-5. doi: 10.1016/j.nut.2009.10.006. Epub 2009 Dec 29.


Diet-induced hyperhomocysteinemia increases amyloid-beta formation and deposition in a mouse model of Alzheimer's disease.

Zhuo JM, Portugal GS, Kruger WD, Wang H, Gould TJ, Pratico D.

Curr Alzheimer Res. 2010 Mar;7(2):140-9.


Hyperhomocysteinemia promotes inflammatory monocyte generation and accelerates atherosclerosis in transgenic cystathionine beta-synthase-deficient mice.

Zhang D, Jiang X, Fang P, Yan Y, Song J, Gupta S, Schafer AI, Durante W, Kruger WD, Yang X, Wang H.

Circulation. 2009 Nov 10;120(19):1893-902. doi: 10.1161/CIRCULATIONAHA.109.866889. Epub 2009 Oct 26.


Mice heterozygous for germ-line mutations in methylthioadenosine phosphorylase (MTAP) die prematurely of T-cell lymphoma.

Kadariya Y, Yin B, Tang B, Shinton SA, Quinlivan EP, Hua X, Klein-Szanto A, Al-Saleem TI, Bassing CH, Hardy RR, Kruger WD.

Cancer Res. 2009 Jul 15;69(14):5961-9. doi: 10.1158/0008-5472.CAN-09-0145. Epub 2009 Jun 30.


Genetic or nutritional disorders in homocysteine or folate metabolism increase protein N-homocysteinylation in mice.

Jakubowski H, Perla-Kaján J, Finnell RH, Cabrera RM, Wang H, Gupta S, Kruger WD, Kraus JP, Shih DM.

FASEB J. 2009 Jun;23(6):1721-7. doi: 10.1096/fj.08-127548. Epub 2009 Feb 9.


Functional rescue of mutant human cystathionine beta-synthase by manipulation of Hsp26 and Hsp70 levels in Saccharomyces cerevisiae.

Singh LR, Kruger WD.

J Biol Chem. 2009 Feb 13;284(7):4238-45. doi: 10.1074/jbc.M806387200. Epub 2008 Dec 12.


Mouse models of cystathionine beta-synthase deficiency reveal significant threshold effects of hyperhomocysteinemia.

Gupta S, Kühnisch J, Mustafa A, Lhotak S, Schlachterman A, Slifker MJ, Klein-Szanto A, High KA, Austin RC, Kruger WD.

FASEB J. 2009 Mar;23(3):883-93. doi: 10.1096/fj.08-120584. Epub 2008 Nov 5.


Active cystathionine beta-synthase can be expressed in heme-free systems in the presence of metal-substituted porphyrins or a chemical chaperone.

Majtan T, Singh LR, Wang L, Kruger WD, Kraus JP.

J Biol Chem. 2008 Dec 12;283(50):34588-95. doi: 10.1074/jbc.M805928200. Epub 2008 Oct 10.


Valproic acid increases expression of methylenetetrahydrofolate reductase (MTHFR) and induces lower teratogenicity in MTHFR deficiency.

Roy M, Leclerc D, Wu Q, Gupta S, Kruger WD, Rozen R.

J Cell Biochem. 2008 Oct 1;105(2):467-76. doi: 10.1002/jcb.21847.


Cystathionine beta-synthase p.S466L mutation causes hyperhomocysteinemia in mice.

Gupta S, Wang L, Hua X, Krijt J, Kozich V, Kruger WD.

Hum Mutat. 2008 Aug;29(8):1048-54. doi: 10.1002/humu.20773.

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